Title: Multiple Coronary Arteriovenous Fistulas to the Coronary Sinus With an Unruptured Coronary Sinus Aneurysm and Restrictive Coronary Sinus Opening to the Right Atrium
Abstract: HomeCirculationVol. 120, No. 12Multiple Coronary Arteriovenous Fistulas to the Coronary Sinus With an Unruptured Coronary Sinus Aneurysm and Restrictive Coronary Sinus Opening to the Right Atrium Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBMultiple Coronary Arteriovenous Fistulas to the Coronary Sinus With an Unruptured Coronary Sinus Aneurysm and Restrictive Coronary Sinus Opening to the Right Atrium Sung-Ho Jung, Won-Chul Cho, Suk Jung Choo, Hyun Song, Cheol Hyun Chung, Joon-Won Kang, Jae-Kwan Song and Jae Won Lee Sung-Ho JungSung-Ho Jung From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author , Won-Chul ChoWon-Chul Cho From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author , Suk Jung ChooSuk Jung Choo From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author , Hyun SongHyun Song From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author , Cheol Hyun ChungCheol Hyun Chung From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author , Joon-Won KangJoon-Won Kang From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author , Jae-Kwan SongJae-Kwan Song From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author and Jae Won LeeJae Won Lee From the Department of Thoracic and Cardiovascular Surgery (S.-H.J., W.-C.C., S.J.C., H.S., C.H.C., J.W.L.), Department of Radiology (J.-W.K.), and Department of Cardiology (J.-K.S.), Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. Search for more papers by this author Originally published22 Sep 2009https://doi.org/10.1161/CIRCULATIONAHA.108.846329Circulation. 2009;120:1138–1140A 37-year-old woman was transferred to our department for surgery. She had experienced intermittent chest discomfort or pain and palpitation during the last 4 years. Chest radiographs showed cardiomegaly (cardiothoracic ratio 0.63). A preoperative ECG showed normal sinus rhythm (Figure 1). Echocardiography showed marked dilatation of the coronary sinus with an intramural thrombus and flow acceleration at its opening (Figure 2). Cardiac catheterization showed no significant coronary artery stenosis but revealed coronary arteriovenous fistulas (CAVFs) from the distal right coronary artery, distal left anterior descending artery, and proximal left circumflex artery draining directly into the coronary sinus (Figure 3). A preoperative computed tomography scan showed a large aneurysm that contained thrombi with a maximal diameter of 73 mm at the outer side of the left ventricular inferior wall (Figure 4). The site of the aneurysm coincided with the coronary sinus and greater cardiac vein. The computed tomography scan was unable to reveal drainage of the thrombosed aneurysm into the right atrium (RA). Magnetic resonance imaging, however, showed a pinpoint opening from the coronary sinus aneurysm to the RA (Figure 5). The right coronary artery was diffusely dilated and was connected to the coronary sinus after forming a small coronary artery aneurysm. Distal collateral vessels from the left anterior descending artery and left circumflex artery were connected directly to the coronary sinus (Figure 6). On the basis of these findings, a final diagnosis of multiple CAVFs to the coronary sinus with a coronary sinus aneurysm and restricted coronary sinus opening was made. Download figureDownload PowerPointFigure 1. Preoperative ECG showing normal sinus rhythm.Download figureDownload PowerPointFigure 2. Preoperative echocardiographic images. Parasternal long-axis view (A) showed marked dilatation of the coronary sinus (CS), which was partially occluded by the thrombus (B). Right ventricular inflow views demonstrated restricted opening of the coronary sinus (C, arrowhead) and flow acceleration (D). Ao indicates aorta; LV, left ventricle; LA, left atrium; and RV, right ventricle.Download figureDownload PowerPointFigure 3. A, Conventional coronary angiography of the right coronary artery system in the right anterior oblique caudal projection showed diffuse dilated right coronary artery draining to the coronary sinus aneurysm (★). B, Coronary angiography of the left coronary artery system showed collateral vessels of the left circumflex artery (arrow) and left anterior descending coronary artery (arrowhead) draining to the coronary sinus aneurysm.Download figureDownload PowerPointFigure 4. Preoperative computed tomography scan. Arrow indicates huge aneurysm containing thrombi. RV indicates right ventricle; LV, left ventricle; and An, aneurysm.Download figureDownload PowerPointFigure 5. In images from the 4-chamber view (A) and vertical long-axis view (B) of cine magnetic resonance examinations, a slitlike opening of the aneurysm to the RA is seen (arrow). In a vertical long-axis image and motion images of the velocity-encoded magnetic resonance examination (C and D) corresponding to Figure 5B, a pinpoint flow signal from the aneurysm to the RA is also seen (arrow). RV indicates right ventricle; LV, left ventricle; and An, aneurysm.Download figureDownload PowerPointFigure 6. Computed tomography scan of the coronary artery reconstruction. This figure shows the right coronary artery (A), left circumflex artery (B), and left anterior descending coronary artery (C) draining to the coronary sinus aneurysm (D).The patient underwent surgical treatment under cardiopulmonary bypass with separate bicaval cannulation. The CAVF site was identified before aortic clamping was performed. On direct observation, the right coronary artery was dilated and tortuous. Numerous collateral vessels were present on the cardiac surface. Of note, a large aneurysm was visible after upward traction of the left ventricular apex (Figure 7A). After the aneurysm was opened, the thrombi (Figure 7B) within were excised completely, and the fistulous site was examined. After the RA was opened, the coronary sinus opening was found to be almost completely occluded. After the coronary sinus opening was widened from the aneurysmal sac, it was possible to identify the main opening site of the coronary fistula at the junction of the coronary sinus and the RA. This site was closed directly with 2 pledgeted mattress sutures from the RA side. The aneurysmal sac was then excised and closed directly with 4-0 polypropylene. The fistulous communication was then ligated at the origin from the right coronary artery with metal clips. We also identified and ligated the fistulous collateral vessel from the left anterior descending artery and left circumflex artery draining into the coronary sinus aneurysm with external metal clips. After inducing cardioplegic arrest, we performed a left-side Maze operation using a CryoCath for paroxysmal atrial fibrillation. The postoperative course was uneventful, and the follow-up computed tomography scan showed a wide connection between the coronary sinus and the RA, as well as a reduction in the size of the coronary sinus aneurysm (Figure 8). The coronary computed tomography scan showed there was no residual blood flow to the fistula. Download figureDownload PowerPointFigure 7. Photograph of intraoperative field. The huge aneurysm can be seen (A), and thrombi can be seen after opening of the aneurysmal sac (B). LV indicates left ventricle; An, aneurysm.Download figureDownload PowerPointFigure 8. Postoperative computed tomography scan showing a wide connection (arrow) between the coronary sinus and the RA and a reduction in size of the coronary sinus aneurysm. RV indicates right ventricle; LV, left ventricle; and CS, coronary sinus.A CAVF is an abnormal connection between a coronary artery and any of the 4 chambers of the heart or any of the great vessels. The right coronary artery and right ventricle are the most common origin and distal connection sites, respectively.1,2 Coronary sinus drainage has been found in 7% of surgical cases and multiple coronary artery fistulas in 5% of patients.1,3 Although the course of this malformation is usually benign, significant complications can occur, such as congestive heart failure, bacterial endocarditis, myocardial ischemia, ventricular arrhythmia, or sudden death.4 In the present case, the coronary sinus dilatation was initially thought to have occurred owing to the multiple connections of the CAVF, which then went on to develop relative stenosis of the coronary sinus opening with flow stasis and mural thrombi. Thereafter, the stenosis of the RA opening may have become aggravated owing to the enlargement of the coronary sinus aneurysm with further aneurysmal change of the coronary sinus.In a few cases, rupture of a coronary artery aneurysm has been reported.4,5 In addition, 1 autopsy case showed coronary sinus rupture with a fistulous connection with the left circumflex artery.6 To the best of our knowledge, the present report is the first to describe the successful surgical correction of multiple CAVF drainage into the coronary sinus combined with an unruptured coronary sinus aneurysm and restricted coronary sinus opening. Left untreated, the coronary sinus aneurysm in the present case may well have ruptured. A left-side Maze procedure was additionally performed to treat the paroxysmal atrial fibrillation while the patient was in normal sinus rhythm.DisclosuresNone.FootnotesCorrespondence to Jae Won Lee, MD, PhD, Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, 388-1 Poongnap-dong, Songpa-gu, Seoul, Republic of Korea. E-mail [email protected]References1 Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula: report of 13 patients, review of the literature and delineation of management. Circulation. 1979; 59: 849–854.CrossrefMedlineGoogle Scholar2 Urrutia SC, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg. 1983; 35: 300–307.CrossrefMedlineGoogle Scholar3 Lowe JE, Oldham HN Jr, Sabiston DC Jr. Surgical management of congenital coronary artery fistulas. Ann Surg. 1981; 194: 373–380.CrossrefMedlineGoogle Scholar4 Bauer HH, Allmendinger PD, Flaherty J, Owlia D, Rossi MA, Chen C. Congenital coronary arteriovenous fistula: spontaneous rupture and cardiac tamponade. Ann Thorac Surg. 1996; 62: 1521–1523.CrossrefMedlineGoogle Scholar5 Misumi T, Nishikawa K, Yasudo M, Suzuki T, Kumamaru H. Rupture of an aneurysm of a coronary arteriovenous fistula. Ann Thorac Surg. 2001; 71: 2026–2027.CrossrefMedlineGoogle Scholar6 Habermann JH, Howard ML, Johnson ES. Rupture of the coronary sinus with hemopericardium: a rare complication of coronary arteriovenous fistula. Circulation. 1963; 28: 1143–1144.CrossrefMedlineGoogle Scholar eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. 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Paparoni F, Algeri E, Degroote P, Richardson M and Lemesle G (2014) A giant right coronary artery related to a small fistula to the coronary sinus, Cardiovascular Revascularization Medicine, 10.1016/j.carrev.2013.08.007, 15:2, (121-124), Online publication date: 1-Mar-2014. Shah S, Teague S, Lu J, Dorfman A, Kazerooni E and Agarwal P (2012) Imaging of the Coronary Sinus: Normal Anatomy and Congenital Abnormalities, RadioGraphics, 10.1148/rg.324105220, 32:4, (991-1008), Online publication date: 1-Jul-2012. Oliver W, Mauermann W and Nuttall G (2011) Uncommon Cardiac Diseases Kaplan's Cardiac Anesthesia: The Echo Era, 10.1016/B978-1-4377-1617-7.00027-3, (675-736), . September 22, 2009Vol 120, Issue 12 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.108.846329PMID: 19770413 Originally publishedSeptember 22, 2009 PDF download Advertisement